Cases reported "Scleromyxedema"

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1/2. scleromyxedema, a positive effect with thalidomide.

    scleromyxedema is a rare dermatological disorder marked by widespread symmetric 2- to 3-mm, firm, waxy, closely spaced papules involving the hands, forearms, face, neck, upper trunk and thighs. The most common extracutaneous manifestation of scleromyxedema is a benign plasma cell dyscrasia. Treatment of scleromyxedema is limited by the lack of long-term results, toxicity and significant adverse side effects. We report a severe case of scleromyxedema who had marked improvement when treated with thalidomide. ( info)

2/2. Nephrogenic systemic fibrosis mimicking inflammatory breast carcinoma.

    Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a newly recognized systemic fibrosing disorder primarily affecting patients with chronic renal failure. patients with skin involvement often develop papules and plaques with peau d'orange surface changes. The lower extremities and trunk are most commonly affected. The most important histologic differential diagnosis is with scleromyxedema. To our knowledge, we report the first case of nephrogenic systemic fibrosis involving the breasts of a 61-year-old woman with end-stage renal disease, clinically mimicking inflammatory breast carcinoma. We propose that nephrogenic systemic fibrosis be considered in the differential diagnosis as a rare possibility when cutaneous changes in the breast suggest inflammatory breast carcinoma in a patient with renal failure. ( info)


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